Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course

Sam Sirotnikov; Louis P. Dehner; José E. Velázquez Vega; Jinjun Cheng

doi:10.1177/10935266241281517

Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course

Sam Sirotnikov, Louis P. Dehner, José E. Velázquez Vega, Jinjun Cheng

Division of Anatomic and Molecular Pathology (AMP)

Research output: Contribution to journal › Article › peer-review

Abstract

Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

Original language	English
Pages (from-to)	68-73
Number of pages	6
Journal	Pediatric and Developmental Pathology
Volume	28
Issue number	1
DOIs	https://doi.org/10.1177/10935266241281517
State	Published - Jan 1 2025

Keywords

BRAF V600E
LCH
LCS
diagnostic criteria
histiocytosis
malignancy

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title = "Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course",

abstract = "Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.",

keywords = "BRAF V600E, LCH, LCS, diagnostic criteria, histiocytosis, malignancy",

author = "Sam Sirotnikov and Dehner, {Louis P.} and {Vel{\'a}zquez Vega}, {Jos{\'e} E.} and Jinjun Cheng",

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AU - Sirotnikov, Sam

AU - Dehner, Louis P.

AU - Velázquez Vega, José E.

AU - Cheng, Jinjun

PY - 2025/1/1

Y1 - 2025/1/1

N2 - Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

AB - Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

KW - BRAF V600E

KW - LCH

KW - LCS

KW - diagnostic criteria

KW - histiocytosis

KW - malignancy

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SN - 1093-5266

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JO - Pediatric and Developmental Pathology

JF - Pediatric and Developmental Pathology

IS - 1

ER -

Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course

Abstract

Keywords

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